64-year-old woman • hot flashes, facial flushing,
excessive sweating, and palpitations • daily
headaches • history of hypertension • Dx?

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doi:doi: 10.12788/jfp.0605

Case Reports

64-year-old woman • hot flashes, facial flushing, excessive sweating, and palpitations • daily headaches • history of hypertension • Dx?

J Fam Pract. 2023 June;72(5):222-224 | doi: 10.12788/jfp.0605

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References

1. Carey RM, Calhoun DA, Bakris GL, et al. Resistant hypertension: detection, evaluation, and management: a scientific statement from the American Heart Association. Hypertension. 2018;72:e53-e90. doi: 10.1161/HYP.0000000000000084

2. Young WF Jr. Diagnosis and treatment of primary aldosteronism: practical clinical perspectives. J Intern Med. 2019;285:126-148. doi: 10.1111/joim.12831

3. Young WF Jr, Calhoun DA, Lenders JWM, et al. Screening for endocrine hypertension: an Endocrine Society Scientific Statement. Endocr Rev. 2017;38:103-122. doi: 10.1210/er.2017-00054

4. Lenders JWM, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002;287:1427-1434. doi: 10.1001/jama.287.11.1427

5. Lenders JW, Duh Q-Y, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2014;99:1915-1942. doi: 10.1210/jc.2014-1498

6. Kimura N, Takayanagi R, Takizawa N, et al. Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma. Endocr Relat Cancer. 2014;21:405-414. doi: 10.1530/ERC-13-0494

7. Thompson LDR. Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol. 2002;26:551-566. doi: 10.1097/00000478-200205000-00002

8. Vaidya A, Hamrahian A, Bancos I, et al. The evaluation of incidentally discovered adrenal masses. Endocr Pract. 2019;25:178-192. doi: 10.4158/DSCR-2018-0565

9. Young WF Jr. Conventional imaging in adrenocortical carcinoma: update and perspectives. Horm Cancer. 2011;2:341-347. doi: 10.1007/s12672-011-0089-z

10. Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019;381:552-565. doi: 10.1056/NEJMra1806651

11. Iñiguez-Ariza NM, Kohlenberg JD, Delivanis DA, et al. Clinical, biochemical, and radiological characteristics of a single-center retrospective cohort of 705 large adrenal tumors. Mayo Clin Proc Innov Qual Outcomes. 2017;2:30-39. doi: 10.1016/j.mayocpiqo.2017.11.002

12. Marty M, Gaye D, Perez P, et al. Diagnostic accuracy of computed tomography to identify adenomas among adrenal incidentalomas in an endocrinological population. Eur J Endocrinol. 2018;178:439-446. doi: 10.1530/EJE-17-1056

13. Pacak K. Preoperative management of the pheochromocytoma patient. J Clin Endocrinol Metab. 2007;92:4069-4079. doi: 10.1210/jc.2007-1720

Pathologic features to look for include capsular/periadrenal adipose invasion, increased cellularity, necrosis, tumor cell spindling, increased/atypical mitotic figures, and nuclear pleomorphism. Radiographic features include larger size (≥ 4-6 cm),¹¹ an irregular shape, necrosis, calcifications, attenuation of 10 HU or higher on noncontrast CT, absolute washout of 60% or lower, and relative washout of 40% or lower.^8,12On MRI, malignant lesions appear hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging.⁹ Fluorodeoxyglucose avidity on PET scan also is indicative of malignancy.^8,9

Treatment for pheochromocytoma is surgical resection. An experienced surgical team and proper preoperative preparation are necessary because the induction of anesthesia, endotracheal intubation, and tumor manipulation can lead to a release of catecholamines, potentially resulting in an intraoperative hypertensive crisis, cardiac arrhythmias, and multiorgan failure.

Metastatic lesions can occur decades after resection, making long-term follow-up critical.

Proper preoperative preparation includes taking an alpha-adrenergic blocker, such as phenoxybenzamine, prazosin, terazosin, or doxazosin, for at least 7 days to normalize the patient’s blood pressure. Patients should be counseled that they may experience nasal congestion, orthostasis, and fatigue while taking these medications. Volume expansion with intravenous fluids also should be performed and a high-salt diet considered. Beta-adrenergic blockade can be initiated once appropriate alpha-adrenergic blockade is achieved to control the patient’s heart rate; beta-blockers should never be started first because of the risk for severe hypertension. Careful hemodynamic monitoring is vital intraoperatively and postoperatively.^5,13Because metastatic lesions can occur decades after resection, long-term follow-up is critical.^5,10

Following tumor resection, our patient’s blood pressure was supported with intravenous fluids and phenylephrine. She was able to discontinue all her antihypertensive medications postoperatively, and her plasma free and urinary fractionated metanephrine levels returned to within normal limits 8 weeks after surgery. Five years after surgery, she continues to have no signs of recurrence, as evidenced by annual negative plasma free metanephrines testing and abdominal/pelvic CT.

THE TAKEAWAY

This case highlights the importance of recognizing resistant hypertension and a potential secondary cause of this disease—pheochromocytoma. Although rare, pheochromocytomas confer increased risk for cardiovascular disease and death. Thus, swift recognition and proper preparation for surgical resection are necessary. Malignant lesions can be diagnosed only upon discovery of metastatic disease and can recur for decades after surgical resection, making diligent long-term follow-up imperative.

CORRESPONDENCE
Nicole O. Vietor, MD, Division of Endocrinology, Walter Reed National Military Medical Center, 8901 Wisconsin Avenue, Bethesda, MD 20889; nicole.o.vietor.mil@health.mil